Alacrima Workup

  • Author: Dan D DeAngelis, MD, FRCS(C); Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 26, 2012
 

Imaging Studies

  • CT scan of the orbits - Aplastic/hypoplastic lacrimal glands
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Procedures

  • Neostigmine testing can be performed as a challenge to evaluate for the presence of secondary tear production.
    • Dosages vary but can be calculated with this formula: [Weight (kg)/70] X 1.5 mg = dose
    • The patient may be reexamined 30-45 minutes later for evidence of tear production. Typically, no secondary tear production is seen in patients with alacrima.
  • Schirmer testing
    • This secretory test is used in the evaluation of patients with alacrima. Testing can be performed to test for basal secretors or for combined basal and reflex secretors. Schirmer test strips are specially prepared Whatman #41 paper, each measuring 35 mm in length and 5 mm in width. A notch is located 5 mm from the end to facilitate bending and placement in the conjunctival fornix. After placement in the fornix, tears advance via capillarity and reflect the tear production of the eye.
    • For basal secretor testing, a drop of topical anesthetic is placed in the conjunctival fornix. The inferior fornix should be dried with a cotton-tipped applicator. The Schirmer test strip is bent at the 5 mm notch and placed in the central lid of each eye. The strips are removed at 5 minutes, and a pen mark is placed at the junction of the wet test strip and the dry test strip. A measurement is made between the mark and the 5 mm notch. A normal value is 10 mm of wetting at 5 minutes, with lower values indicating a dry eye condition. Of asymptomatic patients, 15% have values of less than 3 mm.
    • For basal and reflex secretor testing, no topical anesthetic is used. The inferior fornix should be dried with a cotton-tipped applicator. The test is carried out as outlined above, and the normal median wetting approximates 15 mm, with a range of 10-30 mm. A value of less than 5 mm is abnormally low.
  • Conjunctival and lacrimal gland biopsy specimens may be of value to identify underlying histopathologic findings.
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Histologic Findings

Conjunctiva biopsy specimens reveal hydropic epithelial degeneration. Lacrimal gland biopsy specimens may reveal neuronal degeneration and depletion of dense core vesicles.

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Contributor Information and Disclosures
Author

Dan D DeAngelis, MD, FRCS(C)  Lecturer, Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Ophthalmologist, Department of Ophthalmology and Vision Sciences, Hospital for Sick Children

Dan D DeAngelis, MD, FRCS(C) is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, California Medical Association, Canadian Medical Association, Canadian Ophthalmological Society, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Jeff Hurwitz, MD, FRCS(C)  Director of Ophthalmic Plastic Surgery, Ophthalmologist-in-Chief, Mount Sinai Hospital; Chairman, Professor, Department of Ophthalmology, University of Toronto, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Jorge G Camara, MD  Professor of Ophthalmology, Department of Surgery and Director of Fellowship Training Program in Ophthalmic Plastic and Reconstructive Surgery for Countries Served by the Aloha Medical Mission, University of Hawaii John A Burns School of Medicine

Jorge G Camara, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and American Society of Ophthalmic Plastic and Reconstructive Surgery

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mark T Duffy, MD, PhD  Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Huebner A, Kaindl AM, Knobeloch KP, Petzold H, Mann P, Koehler K. The triple A syndrome is due to mutations in ALADIN, a novel member of the nuclear pore complex. Endocr Res. Nov 2004;30(4):891-9. [Medline].

  2. Akova YA, Demirhan B, Cakmakci S, Aydin P. Pyogenic granuloma: a rare complication of silicone punctal plugs. Ophthalmic Surg Lasers. Jul-Aug 1999;30(7):584-5. [Medline].

  3. Allgrove J, Clayden GS, Grant DB, Macaulay JC. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet. Jun 17 1978;1(8077):1284-6. [Medline].

  4. Babu K, Murthy KR, Babu N, Ramesh S. Triple A syndrome with ophthalmic manifestations in two siblings. Indian J Ophthalmol. Jul-Aug 2007;55(4):304-6. [Medline].

  5. Davidoff E, Friedman AH. Congenital alacrima. Surv Ophthalmol. Sep-Oct 1977;22(2):113-9. [Medline].

  6. Gazarian M, Cowell CT, Bonney M, Grigor WG. The "4A" syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediatr. Jan 1995;154(1):18-23. [Medline].

  7. Hegab SM, al-Mutawa SA. Congenital hereditary autosomal recessive alacrima. Ophthalmic Genet. Mar 1996;17(1):35-8. [Medline].

  8. Kinjo S, Takemoto M, Miyako K, Kohno H, Tanaka T, Katsumata N. Two cases of Allgrove syndrome with mutations in the AAAS gene. Endocr J. Oct 2004;51(5):473-7. [Medline].

  9. Mondino BJ, Brown SI. Hereditary congenital alacrima. Arch Ophthalmol. Sep 1976;94(9):1478-84. [Medline].

  10. Moore PS, Couch RM, Perry YS, Shuckett EP, Winter JS. Allgrove syndrome: an autosomal recessive syndrome of ACTH insensitivity, achalasia and alacrima. Clin Endocrinol (Oxf). Feb 1991;34(2):107-14. [Medline].

  11. Mullaney PB, Weatherhead R, Millar L, Ayyash II, Ayberk H, Cai F, et al. Keratoconjunctivitis sicca associated with achalasia of the cardia, adrenocortical insufficiency, and lacrimal gland degeneration: Keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and adrenocortical function. Ophthalmology. Apr 1998;105(4):643-50. [Medline].

  12. Ornek K, Atilla H, Zilelioglu G. Pediatric alacrima, achalasia, and mental retardation. J AAPOS. Aug 2002;6(4):261-3. [Medline].

  13. Riley CM. Familial autonomic dysfunction. J Am Med Assoc. Aug 23 1952;149(17):1532-5. [Medline].

  14. Sjogren H. The lacrimal secretion in newborn premature and fully developed children. Acta Ophthalmol (Copenh). 1955;33(5):557-60. [Medline].

  15. Smith RS, Maddox SF, Collins BE. Congenital alacrima. Arch Ophthalmol. Jan 1968;79(1):45-8. [Medline].

  16. Uleckas JK, Garel L, Milot J, Mathieu-Millaire F. Orbital CT scan in congenital alacrima. J Pediatr Ophthalmol Strabismus. Mar-Apr 1994;31(2):114-7. [Medline].

  17. Weber A, Wienker TF, Jung M, Easton D, Dean HJ, Heinrichs C, et al. Linkage of the gene for the triple A syndrome to chromosome 12q13 near the type II keratin gene cluster. Hum Mol Genet. Dec 1996;5(12):2061-6. [Medline].

  18. Yoshita T, Kobayashi A, Sugiyama K. Bilateral corneal perforation in an infant with congenital alacrima. J Pediatr Ophthalmol Strabismus. Jul-Aug 2006;43(4):236-8. [Medline].

 
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